Recent outcome of liver transplantation for Budd Chiari syndrome - analysis of the european liver transplant registry (ELTR) and affiliated centres

Background: Maintenance anticoagulation and Transjugular Intrahepatic Portosystemic Shunt (TIPS) have improved management of Budd-Chiari Syndrome (BCS) over the last decades. Most published studies on outcomes of liver transplantation (LT) for BCS in Europe date before these changes.
Methods: Data were obtained from the European Liver Transplantation Registry (ELTR). Age <16, secondary BCS and hepatocellular carcinoma were excluded. Patient (PS) and graft survival (GS) before and after 2000 was compared. Multivariate
Cox regression analysis (with re-transplantation as time-
dependent covariate) identified predictors of PS and GS after 2000. Supplementary data was requested from all ELTR affiliated centres and received from 39.
Results: 811 patients were transplanted for primary BCS between 2000 and 2020. Median age was 37.2y, 60% were female, median MELD was 17 and 29% had high urgency (HU) listing. One-, five- and ten-year PS rates between 2000-2020 were 83%, 76% and 69%, compared to 71%, 66% and 61% for the 293 patients transplanted before 2000 (p<0.001), while GS was 78%, 69%, 62% vs. 63%, 58% and 52%, respectively (p<0.001). Since 2000, BCS recurred in 3% and 12% received a re-transplant. Older recipient age (HR 1.02; 95%CI 1.01-1.04) and higher MELD (HR 1.03; 95%CI 1.01-1.06) were associated with worse PS while HU listing was associated with improved PS (HR 0.57; 95%CI 0.35-0.92). Older donor age was the only independent predictor of worse GS (HR 1.01; 95%CI 1.00-1.02). In n=236 (29%) with additional centre-data, 38% had myeloproliferative disease, 25% received TIPS pre-LT and 82% used anticoagulation post-LT. In these, anticoagulation was the only independent factor associated with PS (HR 0.38; 95%CI 0.15-0.98).Conclusions: LT for BCS results in excellent patient and graft survival. Outcomes have improved since 2000. Older recipient age and higher MELD result in poorer survival. HU listing appears to select patients with most favourable outcome. Long-term anticoagulation seems beneficial. Further validation is needed.